Related Experiment Video
Updated: Aug 11, 2026

Assessment of Chimeric Antigen Receptor T Cell-Associated Toxicities Using an Acute Lymphoblastic Leukemia Patient-Derived Xenograft Mouse Model
Published on: February 10, 2023
Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis
Edda Mittheisz1, Rainer Seidl, Daniela Prayer
1Children's Cancer Research Institute, Vienna, Austria.
Background:
Permanent consequences in Langerhans cell histiocytosis (LCH) are irreversible late sequelae related to the disease that may severely impair the quality of life of survivors. The frequency and pattern of permanent consequences affecting the central nervous system (CNS) remains to be determined.
Procedure:
In this single center study, 25 LCH patients observed for a median time of 10 years 3 months underwent a uniform thorough follow-up program including neuropsychological testing and electrophysiological evaluation.
Results:
Overall permanent consequences were seen in 9 of 25 patients. Intracranial abnormalities were the most frequent including diabetes insipidus (DI) in seven patients, anterior pituitary deficiencies in five patients, and neurodegenerative CNS disease in five patients. No patient had overt neurological symptoms upon neurological evaluation, but psychological testing revealed subtle deficits in short-term auditory memory (STAM) in 14 patients. Brain stem evoked potentials showed abnormalities in four of nine tested patients, all of these four had neurodegeneration on MRI.
Conclusion:
Psychoneuroendocrine sequelae were found in an unexpectedly high number of patients in this single center study. Long-term follow-up focusing on such sequelae are important in LCH survivors, in order to detect early deficits, to monitor the evolution of the disease, and to provide specific support.
Related Concept Videos
Secondary Spinal Cord Injury llI: Pathophysiology
Lysosomal Hydrolases
Huntington Disease l: Introduction
Neurogenesis and Regeneration of Nervous Tissue
Multiple Sclerosis l: Introduction